UNASSIGNED: The clinical features of posterior cortical atrophy (PCA), a rare condition often caused by Alzheimer\'s disease, have been recently defined, while little is known about its neurophysiological correlates.
UNASSIGNED: To describe neurophysiological alterations of the visual pathway as assessed using visual field test (VF), visual evoked potentials (VEP), and electroretinogram (ERG) in PCA patients.
UNASSIGNED: Studies reporting VF, VEPs, and ERG in PCA patients were selected according PRISMA method. Of the 323 articles that emerged from the literature, 17 included the outcomes of interest. To these data, we added those derived from a patient cohort enrolled at our clinic.
UNASSIGNED: The literature review included 140 patients, half of them (50%) presented with homonymous hemianopia or quadrantanopia. VEPs were available in 4 patients (2 normal findings, 1 decreased amplitude, and 1 increased latency) and ERG in 3 patients (substantially normal findings). Our case series included 6 patients, presenting with homonymous lateral hemianopia in 50% and contralateral cortical atrophy. VEPs showed normal amplitude in 66-83% according to the stimulation check, and increased latency in 67% in absence of myelin damage on MRI. Latency was increased in both eyes in 50% and only on one side in the other 50%. Such alterations were observed in patients with more severe and symmetric atrophy. ERG showed normal findings.
UNASSIGNED: Neurophysiological investigations of the visual pathway in PCA are almost absent in literature. Alterations involve both amplitude and latency and can be also monocular. A multiple-point involvement of the optical pathway can be hypothesized.

Posterior cortical atrophy (PCA) is a neurodegenerative syndrome characterised by progressive visuospatial and visuoperceptual impairment. Recent research shows that memory impairment can also occur as an early symptom of the condition and that the impairment can be ameliorated by providing support in the memory recall phase, for example, by presenting a related cue. In Alzheimer\'s disease (AD), which is defined by an amnestic syndrome, memory aids and strategies have been used to help support everyday memory, which in turn can have a positive impact on patient and carer outcomes. Similar support for PCA could be achieved by using memory aids and strategies which help to encode and/or retrieve information, yet there are currently no guidelines for memory strategies that may be suitable in PCA. Due to the central visual disorder that defines PCA, careful consideration is needed when making recommendations.
A scoping review will be conducted of published studies that have assessed memory aids and strategies in people with AD and related dementias where memory is considered a core or supplementary feature, with the aim of distinguishing those that may be suitable or adaptable for PCA. The systematic search will include the electronic databases MEDLINE, PsycINFO and CINAHL, using search terms for dementia and memory aids and strategies identified in pilot searches. Findings will be mapped and described based on methods used, population, clinical data and memory aids and strategies identified.
The scoping review will give an overview of the memory aids and strategies used in people with AD and related dementias and identify characteristics, modality and pragmatics to evaluate their suitability and adaptability for a PCA population. Tailored memory support strategies for people living with PCA could improve memory performance, with knock-on positive effects on patient and carer outcomes.

To provide perspectives on the importance of understanding longitudinal profiles of posterior cortical atrophy (PCA) and report results of a scoping review to identify data and knowledge gaps related to PCA survival and longitudinal clinical and biomarker outcomes.
Thirteen longitudinal studies were identified; all but two had fewer than 30 participants with PCA. Relatively few longitudinal data exist, particularly for survival. In PCA, posterior cortical dysfunction and atrophy progress at faster rates compared to non-posterior regions, potentially up to a decade after symptom onset. Unlike typical AD, PCA phenotype-defined cognitive dysfunction and atrophy remain relatively more severe compared to other regions throughout the PCA course. Select cognitive tests hold promise as PCA outcome measures and for staging. Further longitudinal investigations are critically needed to enable PCA inclusion in treatment trials and to provide appropriate care to patients and enhance our understanding of the pathophysiology of dementing diseases.

Dementia presenting with prominent higher order visual symptoms may be observed in a range of neurodegenerative conditions and is often challenging to diagnose.
OBJECTIVE: To describe cases of progressive dementia presenting with prominent visual cortical symptoms.
METHODS: We conducted a retrospective search of cases of progressive dementia with predominant visual symptoms, seen at our dementia unit from 1996 to 2006.
RESULTS: Twelve patients (5 men, 7 women) were identified, with ages ranging from 49 to 67 years. At the first examination, the duration of the symptoms ranged from one to ten years and the Mini-Mental State Examination scores from 7 to 27. Eleven patients presented with predominant visuospatial symptoms (partial or complete Balint syndrome) and one with visuoperceptive impairment. Other reported manifestations were: constructional apraxia in 11 patients, partial or complete Gerstmann syndrome in ten, ideomotor apraxia in nine, hemineglect or extinction in four patients, alien hand phenomenon in three, and prosopagnosia in one patient. Memory loss was reported by ten patients, but was not the main complaint in any of these cases. Insight was relatively preserved in five patients even after a long period following the onset of symptoms. Six patients developed parkinsonism during evolution. Clinical diagnoses were possible or probable AD in seven patients, cortico-basal degeneration in four, and dementia with Lewy body in one.
CONCLUSIONS: Clinicians should consider this condition especially in presenile patients with slowly progressive higher-order visual symptoms. Although described in association with different conditions, it may also occur in Alzheimer disease.
As demências que se apresentam predominantemente com sintomas visuais associativos podem ser observadas em diferentes condições neurodegenerativas, sendo seu diagnóstico muitas vezes desafiador.
OBJECTIVE: Descrever as principais características clínicas de pacientes com demência progressiva que se apresentam com sintomas visuais proeminentes.
UNASSIGNED: Conduzimos estudo retrospectivo de casos com demência progressiva com sintomas visuais predominantes vistos no nosso ambulatório de demência, no período de 1996 até 2006.
RESULTS: Doze pacientes (5 homens e 7 mulheres) foram identificados, com idades variando entre 49 e 67 anos. Na primeira consulta, duração de sintomas variou de um a dez anos e a pontuação do Mini-Exame do Estado Mental variou de 7 a 27 pontos. Onze pacientes apresentaram sintomas visuoespaciais predominantes (Síndrome de Bálint parcial ou completa) e um apresentou alteração visuoperceptiva. Outras manifestações relatadas foram: apraxia construtiva em 11 pacientes, síndrome de Gerstmann parcial ou completa em 10 pacientes, apraxia ideomotora em nove, heminegligência ou extinção em 4 pacientes, fenômeno da mão alienígena em 3 e prosopagnosia em um paciente. Queixa de perda de memória foi referida em 10 pacientes, mas em nenhum deles como queixa principal. Juízo crítico era preservado em cinco pacientes até estágios moderados da doença. Seis pacientes desenvolveram parkinsonismo ao longo da evolução. O diagnóstico clínico foi DA possível ou provável em sete pacientes, degeneração corticobasal em quatro e demência com corpos de Lewy em um paciente.
UNASSIGNED: Os clínicos devem ter em mente essa condição especialmente em pacientes pré-senis com queixas visuais complexas e lentamente progressivas. Apesar de descritas em diferentes condições, pode ocorrer na doença de Alzheimer.